According to the recent 2012 Chapel Hill Consensus Conference, ANCA-associated vasculitis is classified in the group of small vessel vasculitis. Pauci-immune necrotizing crescentic glomerulonephritis is the morphological hallmark of ANCA-associated vasculitis. Crescentic damage of the glomerular tuft is characterized by macrophage accumulation through vascular cell adhesion molecule-1 (VCAM-1) activation. Macrophages have a key role in chronic progression of renal damage due to production of substances involved in matrix remodelling [transforming growth factor-beta (TGFβ)]. Diffuse interstitial infiltration of T and B lymphocytes and macrophages is another frequent morphological feature. Tubulitis is considered an important marker of worse prognosis. Unfortunately, the renal changes on long-term follow-up are largely unknown due to the small number of studies with repeat biopsy in these disorders. Although a standardized score for renal biopsies was developed previously, a final histopathologic classification is still lacking. The European Vasculitis Study Group (EUVAS) proposed a classification system based on glomerular pathology as assessed by light microscopy.

Ferrario, F., Vanzati, A., Pagni, F. (2013). Pathology of ANCA-associated vasculitis. CLINICAL AND EXPERIMENTAL NEPHROLOGY, 17(5), 652-658 [10.1007/s10157-012-0701-8].

Pathology of ANCA-associated vasculitis

PAGNI, FABIO
2013

Abstract

According to the recent 2012 Chapel Hill Consensus Conference, ANCA-associated vasculitis is classified in the group of small vessel vasculitis. Pauci-immune necrotizing crescentic glomerulonephritis is the morphological hallmark of ANCA-associated vasculitis. Crescentic damage of the glomerular tuft is characterized by macrophage accumulation through vascular cell adhesion molecule-1 (VCAM-1) activation. Macrophages have a key role in chronic progression of renal damage due to production of substances involved in matrix remodelling [transforming growth factor-beta (TGFβ)]. Diffuse interstitial infiltration of T and B lymphocytes and macrophages is another frequent morphological feature. Tubulitis is considered an important marker of worse prognosis. Unfortunately, the renal changes on long-term follow-up are largely unknown due to the small number of studies with repeat biopsy in these disorders. Although a standardized score for renal biopsies was developed previously, a final histopathologic classification is still lacking. The European Vasculitis Study Group (EUVAS) proposed a classification system based on glomerular pathology as assessed by light microscopy.
Articolo in rivista - Articolo scientifico
Nephropathology
English
23-nov-2012
2013
17
5
652
658
none
Ferrario, F., Vanzati, A., Pagni, F. (2013). Pathology of ANCA-associated vasculitis. CLINICAL AND EXPERIMENTAL NEPHROLOGY, 17(5), 652-658 [10.1007/s10157-012-0701-8].
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/10281/39536
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